Adenotonsillar pathology in mucopolysaccharidoses - lysosomal storage predominates in paracortical CD63+ cells
The result's identifiers
Result code in IS VaVaI
<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00064173%3A_____%2F24%3A43926102" target="_blank" >RIV/00064173:_____/24:43926102 - isvavai.cz</a>
Alternative codes found
RIV/00216208:11110/24:10469458 RIV/00216208:11120/24:43926102 RIV/00216208:11130/24:10469458 RIV/00064203:_____/24:10469458 RIV/00064165:_____/24:10469458
Result on the web
<a href="https://doi.org/10.1007/s00428-023-03662-y" target="_blank" >https://doi.org/10.1007/s00428-023-03662-y</a>
DOI - Digital Object Identifier
<a href="http://dx.doi.org/10.1007/s00428-023-03662-y" target="_blank" >10.1007/s00428-023-03662-y</a>
Alternative languages
Result language
angličtina
Original language name
Adenotonsillar pathology in mucopolysaccharidoses - lysosomal storage predominates in paracortical CD63+ cells
Original language description
Despite the adenoids are regularly removed in patients with mucopolysaccharidoses (MPS), the underlying tissue and cellular pathologies remain understudied. We characterized an (immuno)histopathologic and ultrastructural phenotype dominated by lysosomal storage changes in a specific subset of adenotonsillar paracortical cells in 8 MPS patients (3 MPS I, 3 MPS II, and 2 MPS IIIA). These abnormal cells were effectively detected by an antibody targeting the lysosomal membrane tetraspanin CD63. Important, CD63+ storage vacuoles in these cells lacked the monocytes/macrophages lysosomal marker CD68. Such a distinct patterning of CD63 and CD68 was not present in a patient with infantile neurovisceral variant of acid sphingomyelinase deficiency. The CD63+ storage pathology was absent in two MPS I patients who either received enzyme-replacement therapy or underwent hematopoietic stem cells transplantation prior the adenoidectomy. Our study demonstrates novel features of lysosomal storage patterning and suggests diagnostic utility of CD63 detection in adenotonsillar lymphoid tissue of MPS patients.
Czech name
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Czech description
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Classification
Type
J<sub>imp</sub> - Article in a specialist periodical, which is included in the Web of Science database
CEP classification
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OECD FORD branch
30109 - Pathology
Result continuities
Project
<a href="/en/project/LX22NPO5107" target="_blank" >LX22NPO5107: National institute for Neurological Research</a><br>
Continuities
I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace
Others
Publication year
2024
Confidentiality
S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů
Data specific for result type
Name of the periodical
Virchows Archiv
ISSN
0945-6317
e-ISSN
1432-2307
Volume of the periodical
484
Issue of the periodical within the volume
1
Country of publishing house
DE - GERMANY
Number of pages
6
Pages from-to
135-140
UT code for WoS article
001074569600001
EID of the result in the Scopus database
2-s2.0-85173091077